Hydatid cyst is a zoonotic infection caused by the larval stage of the worm Echinococcus granulosus. Although any organ could be infected, the involvement of the bone, brain and heart are uncommon. The brain involvement Prevalence varies in the hydatid disease. We present a 27-year-old man with 10 years history of Behcet disease and with an unusual occurrence of brain hydatidosis, diagnosed by MRI and histological findings. Removal of the cyst by surgery was successfully performed and Albendazole was administered for 4 weeks after the surgery (400 mg two times daily) accompanied MLP.